Devic’s Neuromyelitis Optica: A Case with Mitochondrial DNA Mutations

evant pleocytosis is a major supportive criteria. Oligoclonal bands (OBs) have been reported in 22.7% of patients. Similar to MS, OBs may appear during the course of the disease but at variance with MS, they may also disappear [6] . Most authors believe that these features support the concept that DNO is a separate syndrome [7–10] . Compared with MS, DNO affects more frequently females, subjects of an older age, and leads to more severe neurological impairment [1, 3] . Demyelination and cavitation of optic nerves and spinal cord, necrosis of both gray and white matter, lack of infl ammatory infi ltrate, vascular hyalinization and fi brosis are the most relevant pathological fi ndings [11] . DNO can be associated with autoimmune diseases such as systemic lupus erythamatosus, Sjogren syndrome and autoimmune thyroiditis [8–10, 12, 13] . Detection of autoantibodies (antithyroid, antiDNA, anticardiolipin) is frequent [8–10] . However, there is only one case report about DNO associated with a mitochondrial DNA mutation [14] . We herein report the second case with r-DNO and concomitant mutation of mitochondrial DNA.